Monday, March 11, 2013

Pancreatic cancer risk in PJS - report from Netherlands

This recent report from
the Netherlands follows 144 PJS patients for a combined total of 5640 person
years. Seven patients developed pancreatic cancer and four patients were
diagnosed with biliary duct or ampullary cancer.

From this data, the
authors were able to estimate the risk of pancreatico-biliary cancer as 32% by
age 70. This is 96 times the rate of pancreatic cancer for a person in the
general population.

The authors also note in
Table 1, Baseline characteristics of the Dutch PJS Cohort, that 48 of their 144
patients (33%) had died at the median age of 46 years and the median age of the
90 surviving patients was 37 years old. (note, six patients were lost to
follow-up).

Based on the results of
our current study, we recommend that PJS patients should be offered
surveillance regardless of family history for PC, since all subjects with PC in
our series had a negative family history of PC. Although the median age of PC onset
in our cohort was 54 years, we propose that surveillance starts at the age of
30 years. This suggestion is based on the fact that two patients in our series
developed cancer in the pancreatico-biliary region at a very young age (ages 35
and 36).

70 years, with a relative
risk of 96 (95% CI 53 to 174; p<0 .001="" o:p="">0>

CONCLUSIONS: PJS patients
have a highly increased risk for pancreatico-biliary cancer. Therefore,
patients are eligible for surveillance within well defined research programmes
to establish the benefit of such surveillance.